[Diffuse intestinal lymphangiectasia].
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INTRODUCTION: Primary intestinal lymphangiectasia (Waldmann's disease) lead to a protein-losing enteropathy due to lymph leak into intestinal tract. EXEGESIS: A 28-year-old woman presented a bilateral lower limb lymphedema. Laboratory examination showing lymphopenia, hypoalbuminemia, hypogammaglobulinemia suggested the diagnosis of primary intestinal lymphangiectasia. Gastroscopy was normal and second duodenum biopsies were negative. Videocapsule endoscopy gave evidence of intestinal lymphangiectasia of the small bowel. CONCLUSION: Videocapsule endoscopy may be proposed to confirm intestinal lymphangiectasia and to precise their localization when gastroscopy is not conclusive.
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A "halo sign" has been described in patients with Crohn disease, ulcerative colitis, radiation enteritis, ischemic colitis, and pseudomembranous colitis. This sign is characterized by an inner ring of low CT attenuation surrounded by a higher attenuation outer ring. We present a patient with primary intestinal lymphangiectasia in whom CT demonstrated a halo sign correlated with mucosal biopsy.
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A 21-year-old man with diarrhea and edema was admitted to our hospital and diagnosed with protein-losing enteropathy caused by primary intestinal lymphangiectasia. He was placed, in turn, on a low-fat diet, an elemental diet, and, subsequently, fasting therapy with total parenteral nutrition (TPN) support. However, his symptoms were not relieved, but, rather were exacerbated. On the 45th day of hospitalization, octreotide therapy was initiated. After 2 weeks of treatment, his clinical symptoms, as well as hypoproteinemia and hypoalbuminemia, gradually became alleviated. The improvement was confirmed in terms of scintigraphy, endoscopy, and histology of the duodenum. The patient remained healthy until 6 months after the commencement of octreotide treatment, when he discontinued octreotide at his own discretion, at which point the symptoms recurred. Resumption of the drug, however, again brought about remission, which has continued until the present, March 2000. Thus, octreotide therapy is one modality which may be useful for refractory primary intestinal lymphangiectasia.
A case of primary intestinal lymphangiectasia is presented in which multiple blind peroral jejunal biopsies were unable to document any abnormality, despite strongly suggestive clinical history and radiographic findings. Endoscopically directed biopsy was necessary to document the characteristic pathologic lesion. This report documents the importance of endoscopy in the diagnosis of intestinal lymphangiectasia when clinical history is suggestive of intestinal lymphangiectasia but standard small bowel biopsy fails to show any abnormality.
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Zellweger cerebrohepatorenal syndrome (ZWCHRS) is an autosomal-recessive disease, characterized by the absence or profound deficiency of peroxisomes. We report a case of ZWCHRS with intestinal lymphangiectasia, observed as an autopsy finding. This combination is previously unreported.
A 23-year-old woman with congenital intestinal lymphangiectasia developed a progressive spinocerebellar syndrome and neuropathy. The clinical findings were typical of vitamin E deficiency, which was documented in the patient. Intestinal lymphangiectasia is an additional vitamin E-deficient disease that may cause this neurologic syndrome.
The authors report a case of localised intestinal lymphangiectases in a segment of jejunum with the unusual presentation of an acute perforation. The anatomical findings did not provide a satisfactory aetiopathogenic explanation, although the most likely hypothesis is a past history of abdominal irradiation. The mechanism of the perforation also remains unclear and the presence of microscopic lesions in the wall of the remaining small intestine raises the problem of the long-term prognosis.
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